Rituximab for the treatment of IgG4-related orbital disease: experience from five cases.

PURPOSE: To review the clinical efficacy and safety of rituximab for treatment of IgG4-related orbital disease (IgG4-ROD). DESIGN: Retrospective multicentre interventional case series. METHODS: Chart review for five cases of biopsy-confirmed IgG4-ROD (IgG4+>10/HPF, ratio of IgG4+/IgG+>40%) treated with rituximab. Information retrieved included the dosing schedule, adverse events and the magnitude, temporality, and duration of the clinical response. RESULTS: All cases of IgG4-ROD were either steroid dependent or steroid resistant. Rituximab doses for induction therapy included two doses of 1000 mg at 2-weekly intervals, and four doses at 375 mg/m(2) at weekly intervals. Two months after starting rituximab, three cases achieved complete clinical resolution and two cases achieved partial clinical resolution. Complete radiological resolution occurred in one case, and partial radiological resolution in three cases. Three cases received rituximab maintenance therapy and one case was commenced on mycophenolate. No relapse occurred during a mean follow-up of 33 months (range: 7-65 months). One disease relapse occurred when the dosing interval of rituximab maintenance therapy was extended to 6-monthly intervals; remission was swiftly achieved with rituximab reinduction therapy. The only adverse effects reported were one episode of fatigue lasting 1 week and two episodes of orbital discomfort. CONCLUSION: Rituximab may be an effective treatment option for IgG4-ROD that is steroid dependent or steroid intolerant. Rituximab therapy resulted in swift clinical and radiological improvement, many months free of relapse, and few side effects.

Microsporidial keratoconjunctivitis after HAART.

A 44-year-old man presented with bilateral punctate corneal epitheliopathy complaining of worsening discomfort and photophobia over the previous several days. He was HIV positive, had a recent CD4 count of 4 x 10(6), and had started on highly active antiretroviral therapy (HAART) 14 days prior. Failure to respond to lubricant therapy with worsening of the epitheliopathy over the following week led to corneal biopsy and diagnosis of corneal microsporidiosis. Investigations revealed that he remained anergic and that his CD4 count had not changed. However, his viral load had decreased by at least 0.9 log10 units since HAART intiation. Therapy with albendazole led to complete resolution of his pre-existing symptoms of nasal congestion and epistaxis, as well as all recently occurring ocular signs and symptoms. It was concluded that the microsporidiosis was a pre-existing opportunistic infection, whose presence was unmasked by a form of immune restoration induced by HAART.

Coexistent adenoviral keratoconjunctivitis and Acanthamoeba keratitis.

A 17-year-old youth presented with bilateral follicular conjunctivitis and nummular subepithelial corneal infiltrates. Failure of this to settle in an outpatient setting led to corneal scraping with microscopy and culturing for bacteria, fungi, Herpes simplex, adenovirus and Acanthamoeba as an inpatient. Polymerase chain reaction analysis of corneal cells was positive for adenovirus, and culture on live Escherichia coli-coated agar plates was positive for Acanthamoeba by phase contrast microscopy on day two. We conclude that Acanthomoeba infection can complicate adenoviral keratoconjunctivitis. This observation is in keeping with previously reported modes of infection by Acanthamoeba, whereby any epithelial breach seems to allow inoculation of the eye by this opportunistic organism.